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α1-抗胰蛋白酶缺乏基因:银屑病患者亚组中的促成性缺陷?

Alpha 1-antitrypsin deficiency genes: contributory defect in a subset of psoriatics?

作者信息

Lipkin G, Galdston M, Kueppers F

出版信息

J Am Acad Dermatol. 1984 Oct;11(4 Pt 1):615-9. doi: 10.1016/s0190-9622(84)70216-7.

Abstract

Since proteolytic processes are prominent in psoriasis, sera of forty-five psoriatics were examined for alpha 1-antitrypsin (alpha 1-AT) phenotype and eighteen sera, for alpha 1-AT content and function. Five sera (11.1%) had heterozygous phenotypes (2 MZ and 3 MS), a prevalence of Z and S variants similar to that reported in nonpsoriatic populations. Two of three variants examined for content and function exhibited marked reductions. Since MZ heterozygotes are almost always, and MS phenotypes sometimes, associated with decreased serum alpha 1-AT levels, and since Z and MZ phenotypes are associated with increased hepatic fibrosis or cirrhosis, these variants may be relevant to problems of spontaneous fibrosis or methotrexate-induced hepatotoxicity in psoriasis. alpha 1-AT deficiency may also contribute to guttate flares with infection and to increased O-2 . production by psoriatic sera-stimulated polymorphonuclear leukocytes (PMNs). Although no evidence exists that psoriasis is more prevalent among persons with hypomorphic alpha 1-AT phenotypes, such defects may contribute to severity of inflammation and hyperplasia.

摘要

由于蛋白水解过程在银屑病中很突出,对45例银屑病患者的血清进行了α1-抗胰蛋白酶(α1-AT)表型检测,对18例血清进行了α1-AT含量和功能检测。5例血清(11.1%)具有杂合表型(2例MZ和3例MS),Z和S变异体的流行率与非银屑病人群报道的相似。检测的三种变异体中的两种在含量和功能上表现出明显降低。由于MZ杂合子几乎总是与血清α1-AT水平降低相关,MS表型有时也与之相关,并且由于Z和MZ表型与肝纤维化或肝硬化增加相关,这些变异体可能与银屑病中自发纤维化或甲氨蝶呤诱导的肝毒性问题有关。α1-AT缺乏也可能导致点滴状皮疹伴感染以及银屑病血清刺激的多形核白细胞(PMN)产生更多的超氧阴离子。虽然没有证据表明银屑病在α1-AT表型减退的人群中更普遍,但这种缺陷可能会加重炎症和增生的严重程度。

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